This patient presented in his 20s with 2 months of progressive headaches and was found to have a right cerebellar cystic mass.

20x

40x

Hemangioblastoma (WHO grade I):
"Uncertain" Histogenesis

•Various
cell lineages such as vascular, glial, neural, fibrohistiocytic, and smooth
muscle/myofibroblastic have been proposed for the so-called stromal cells,
which are thought to represent the neoplastic component of these lesions

-Neg
for endothelial markers

-CD133,
VEGF positive, Epo/EpoR coexpression'

-GFAP
positive cells may result from phagocytosis

•The
VHL gene is expressed particularly in Purkinje cells of the cerebellum
(mutations may be inherited or somatic).

•VHL
mutation may lead to developmental arrest of angioblasts.Pluripotent neoplastic angioblasts may be the
cell of origin.

The patient was a 35 year old woman who apparently suffered a cardiac event at home. She arrived at the hospital obtunded with minimal brainstem reflexes. Two days after admission she developed V-fib and became completely unresponsive. An MRI at this time showed severe anoxic changes. She was pronounced dead 12 days after the initial event.